Upper Egyptian outcome of Kasai portoenterostomy in biliary atresia: A cross-sectional retrospective study

Abstract

Background: Kasai portoenterostomy (KPE) is the key standard operation for biliary atresia (BA). Age at operation is an important factor that affects the outcome of KPE. The aim of the study is to evaluate the short-term outcome of KPE for biliary atresia infants in Upper Egypt.

Patients and methods: this was a cross-sectional, multicenter retrospective study done in three university hospitals in Upper Egypt. The data was collected from January 1, 2019 to September 30, 2021. All infants diagnosed with BA and managed by KPE were included in the study. The collected data were demographic data (age in months or days, sex, residence, parents' consanguinity, and parents' smoking status), history of present illness (the date of first symptoms, fecal color, skin itching, and associated anomalies), and general and abdominal examinations (liver size, skin, and sclera color).

Results: The total number of patients was 45 infants. Two-thirds of the cases were females. The mean age of KPE was 99 ± 13 days. The mean duration of jaundice clearance and resolution after surgery was 12.3 ± 5.5 weeks in 64.4 % of cases. During the five-year follow-up period, 26 (57.8 %) patients were still alive with native livers. Parental smoking, whether maternal or paternal, prenatal or postnatal, was present in 95.5 % of patients. A massive decrease in ALP level in the 1st postoperative week is an excellent predictor of a good outcome with a cutoff value of 560 U/L. Prophylaxis against developing cholangitis is important for the outcome.

Conclusion: Kasai portoenterostomy is an important operation for biliary atresia infants, whatever the age, under or above 4 months old. Kasai portoenterostomy increases the survival rate of native liver infants who suffer from BA. Jaundice clearance after KPE for BA can be predicted using postoperative AST, ALP, and GGT, as these enzymes decline dramatically at the end of the 3rd postoperative month. Unfortunately, the infants in this study had delayed diagnoses. To address this, targeted interventions such as continuing medical education for local doctors, improved transportation infrastructure, and financial support programs are needed to enhance early diagnosis. Despite the delay in the surgical intervention and the older infant's age, the results were good.

Clinicaltrials: gov Identifier: NCT05925543. Kasai portoenterostomy operation. in Biliary Atresia - Clinical Trials Registry - ICH GCP. The Registration Date was June 29, 2023.

Keywords: Cholangitis; Cholestasis; Neonatal jaundice; Short-term outcome; Survival with native liver; Upper Egypt.